CLEFT LIP and PALATE

ANATOMY

• Cleft Lip

– anterior to the INCISIVE FORAMEN and may also involve the alveolar process

• Cleft Palate

– Primary cleft palate

• failure of fusion of median and lateral palatine processes

– Secondary cleft palate

• failure of fusion of lateral palatine processes

ANATOMY

• Submucous cleft palate (SMCP)

– occult cleft of the soft palate

– classic clinical triad:

1. bifid uvula

2. notching of the hard palate

3. zona pellucida – thinned area of soft palate containing only mucosa due to

– levator veli palatini muscles INSERTING ON HARD PALATE

MUSCLES 1. Levator veli

palatini

2. Tensor veli palatini

3. Palatoglossus

4. Palatofarengeus

5. Stylofarengeus

6. Superior konstrüktörler

7. Uvula

3

5

6

styloglossus

1

2

3

CLASSIFICATION

• Cleft Lip

CLASSIFICATION

• Cleft Palate

PREVELANCE

• Cleft lip with or without cleft palate (CL±CP)

– Ethnicity/Sex

• 1:1000 Caucasians

• 1:2000 African-Americans

• 1:500 Asian

• 2:1 males:females

• Cleft of palate alone (CP)

– 1:2000 (all ethnicity)

– 1:2 males:females

PREVELANCE

ETIOLOGY

• Multifactorial combination

– heredity with or without environmental factors

• Teratogenic agents

– e.g. phenytoin, alcohol

• Nutritional factors may contribute

– folate deficiency

• Syndromic

– 3% of CL±CP are

EMBRYOLOGY

• Cleft lip with palate forms at 4-6 weeks due to lack of

– mesenchymal penetration(merging)

– fusion

• Isolated cleft palate forms later at 7-12 weeks

– lack of fusion

PATHOPHYSIOLOGY AND FUNCTIONAL DEFICITS

• Cleft lip

– Inability to form fluid and air seal in eating or speech

– Malocclusion as a result of intrinsic deformities of alveolar process and teeth

– Lack of continuity of skin, muscle and mucous membrane of lip with

– associated nasal deformity and nasal obstruction

– Deformity

PATHOPHYSIOLOGY AND FUNCTIONAL DEFICITS

• Cleft palate • Inability to separate nasal from oral cavity so that

air and sound escape through nose in attempted speech

• Feeding impaired by loss of sucking due to inability to create intra-oral negative pressure

• Loss of liquids and soft foods through nose due to common nasal-oral chamber

• Middle ear disease and chronic otitis media due to Eustachian tube Dysfunction

PATHOPHYSIOLOGY AND FUNCTIONAL DEFICITS

• Cleft palate

• May be associated with Pierre-Robin sequence

– cleft palate

– micrognathia

– glossoptosis

TEAM CONCEPT

• Because of multiple problems – plastic surgeon

– orthodontist

– dentist,

– geneticist

– pediatrician

– speech therapist

– audiologist

– social worker

– psychologist.

TIMING OF SURGICAL INTERVENTION

• Cleft Lip – Most common 10 weeks of age.

• RULE OF 10’S” – 10 weeks of age

– Hgb 10

– 10 lbs

• Range of cleft lip repair varies from – 0-3 months of age in full-term, otherwise healthy,

infant.

TIMING OF SURGICAL INTERVENTION

• Cleft Palate

– Before purposeful sounds made

– 9-12 mos

– Depending upon health of infant, extent of cleft

• certainly before 18 MONTHS OF AGE if possible

TIMING OF SURGICAL INTERVENTION

• Cleft Nasal Deformity – Most centers perform

• PRIMARY CORRECTION at the time of lip repair

– Secondary rhinoplasty at • preschool age (4-5 years)

• Alveolar cleft – Most centers perform secondary bone grafting at the

stage of mixed dentition • 9-12 years of age • just before eruption of the permanent canine which is often

affected by the cleft

TIMING OF SURGICAL INTERVENTION

• Dentofacial skeletal abnormality

– In most cleft patients, this manifests as

• MAXILLARY RETRUSION/HYPOPLASIA

– In 25% of cleft patients

• orthognathic surgery (jaw-straightening procedure)

• to correct a MALOCCLUSION (abnormal bite).

– Orthognathic surgery CAN ONLY BE performed in skeletally

mature individuals

• 14-16 years of age, women

• 17-19 years of age, men

TIMING OF SURGICAL INTERVENTION

• Dentofacial skeletal abnormality

– With the advent of craniofacial distraction

• surgical intervention can be performed earlier

• both patient and parents must be advised that the

growing child may

– “outgrow” the correction

– necessitating a repeat procedure.

PRINCIPLES OF REPAIR

• Cleft Lip

– 3 months

– Repair of skin, muscle and mucous membrane to restore complete continuity of lip, symmetrical length and function

– Simultaneous repair of both sides of a bilateral cleft lip

– Preference for primary nasal reconstruction at time of lip repair

PRINCIPLES OF REPAIR

• Cleft Lip

– In wide clefts (>10mm)

• presurgical orthodontics

– palatal appliance

– nasoalveolar molding may be indicated

• cleft lip adhesion

– surgery to initially bring lip segments together, followed by definitive repair of lip 3 months later

PRINCIPLES OF REPAIR

• Cleft Palate

– 9-12 months

– One stage repair of both hard and soft palate

• Aveolar cleft

– 6-12 years

– At the time of eruption of permanent canine

teeth

SECONDARY REPAIR

• Cleft Lip

– Orthognathic Lefort I osteotomy

• for maxillary hypoplasia

• 16 years of age

– Secondary rhinoplasty

• 16-18 years of age

SECONDARY REPAIR

• Cleft Palate

– Correction of

• VELOPHARYNGEAL INADEQUACY

• nasal escape air due to remaining palatal defect

• 4-6 years of age

– Repair of any oronasal palatal fistula